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Choroid plexus tumor (CPT)

Every year, approximately 4 children in the Netherlands are diagnosed with a choroid plexus tumor. There are three types of choroid plexus tumors: the choroid plexus papilloma and the atypical choroid plexus papilloma, both of which usually remain in one place, and the malignant choroid plexus carcinoma which can spread. The papilloma occurs mainly in very young children (under 3 years old). The carcinoma can occur at all ages, but more often in children in the upper grades of primary school.

Causes

Choroid plexus tumors arise from plexus cells; these are cells that line the walls of the ventricles (fluid-filled cavities) of the brain and produce cerebrospinal fluid. One of those cells starts to rapidly divide and forms a tumor. The carcinoma is more common in children with Li-Fraumeni syndrome, a congenital syndrome that increases the risk of developing tumors.

Symptoms

Most children suffer from headaches, nausea and vomiting. This is due to increased pressure in the brain: because the tumor continues to grow, there is less room and the cerebrospinal fluid cannot drain away. Other symptoms include abnormal eye movements, poor vision, poor walking ability and sometimes epilepsy. In very young children the skull is not yet closed and the excess cerebrospinal fluid accelerates head growth. They suffer less from increased pressure.

How choroïd plexus tumors diagnosed?

The purpose of an MRI scan is to locate the position of the tumor. This is followed by an operation to remove the whole tumor or as much of the tumor as possible. The pathologist examines the tumor tissue under the microscope to identify the abnormality in the genetic material (DNA) of the tumor. That tells us what type of choroid plexus tumor your child has and we can determine the treatment.

Treatment

Children with a CPT are treated according to a protocol drawn up by national and international experts. In the event of a choroid plexus papilloma, it is usually sufficient for the neurosurgeon to remove the tumor. An operation also suffices for an atypical papilloma in children over the age of 3.
A particular type of atypical papilloma plexus has been known to grow back and even spread in children under the age of 3. Those children therefore receive additional chemotherapy if the tumor returns. In most instances, the tumor then disappears completely and does not recur.
Children with choroid plexus carcinoma receive additional chemotherapy and radiotherapy treatment after surgery. Children under 3 years old with a carcinoma do not undergo radiotherapy due to the risk of damage to the healthy brain tissue. They receive long-term chemotherapy treatment to ensure that the tumor disappears or remains dormant until they are old enough to receive radiotherapy.

Chance of recovery

The majority of children with a choroid plexus papilloma and almost all children over 3 years of age with an atypical choroid plexus papilloma are cured.

All children under the age of 3 with a certain atypical choroid plexus papilloma are also cured if they receive chemotherapy on time.

Choroid plexus carcinoma does not respond very well to chemotherapy and radiotherapy in about 50% of the children, in which case the tumor cannot be brought under control. However, there are certainly children who recover from choroid plexus carcinoma.

Questions?

Children and adolescents with a choroid plexus tumor are treated in the neuro-oncology department.